Abstract
Intravascular papillary endothelial hyperplasia (IPEH) is a rare benign non-neoplastic vascular lesion. A typical presentation consists of a subcutaneous nodule that may simulate other clinical entities. Presentation in the oral cavity is uncommon. It is thought to develop as an abnormal proliferative reaction of endothelial cells in a process of impaired thrombogenesis. When endothelial proliferation occurs, a differential diagnosis with a soft tissue sarcoma, in particular an angiosarcoma, should be performed. We report a case of a 68-year-old female patient who presented with a lesion on the upper lip of 3 months' duration. Surgical resection revealed an IPEH. 1 year later, the patient showed a local recurrence requiring excision with clear margins. Pathological and immunohistochemical features can help us distinguish these lesions from those requiring more aggressive treatment. The gold standard is surgical resection with clear margins. Accurate preoperative diagnosis is essential to avoid overtreatment. Emphasis should be placed on clinical, radiological and histological studies.